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Letters

Granulomatosis with polyangiitis

(Wegeners): An alternative name
for Wegeners granulomatosis
The Boards of Directors of the American College of
Rheumatology (ACR), American Society of Nephrology (ASN)
and the European League Against Rheumatism (EULAR) have
recommended a gradual shift from honoric eponyms to disease-descriptive or aetiology-based nomenclature.
The leadership of these three organizations tasked an international group of senior academicians expert in the care of patients
with vasculitis and engaged in research in the eld to provide the
medical community with proper descriptive terms instead of the
names for Wegeners granulomatosis, ChurgStrauss syndrome,
and Behets syndrome. The move towards a vasculitis terminology based on pathology, rather than historical reference, was
triggered by evidence that Dr Friedrich Wegener was a member
of the Nazi party before and during World War II.1
As the rst step towards a vasculitis nomenclature that is
free of eponyms, the authors of this article held a meeting on 7
November 2010 and reached consensus on an alternative name
for Wegeners granulomatosis. As physicians whose clinical and
research work focuses on vasculitis, we represent the diverse
opinions of our international colleagues within the multiple
medical specialties that have strong interests in vasculitis. This
article announces the newly proposed name, outlines the reasons for seeking a new disease name, and explains the rationale
for the proposed name.
The alternative name for Wegeners granulomatosis is granulomatosis with polyangiitis (Wegeners) which can be abbreviated as
GPA. The parenthetical reference to Wegeners will be phased
out after several years as the new usage becomes more widely
known.
Granulomatosis with polyangiitis was initially described by
Klinger in 1931 as a variant of polyarteritis nodosa, and then in
greater detail as a separate syndrome by Wegener in two articles
appearing in 1936 and 1939.24 The term Wegeners granulomatosis
was introduced into the English-language literature by Drs Godman
and Churg in 1954.5 Granulomatosis with polyangiitis has previously
been proposed as an alternative for Wegeners granulomaotsis.6
We recognise the difculty inherent in seeking a replacement term for a long-established disease name for this complex
multisystem illness with highly variable clinical presentations.
Although this replacement term is neither perfect nor encompasses all aspects of the pathophysiology and clinical spectrum
of the disease, the new term is nonetheless t for the intended
purpose for several important reasons: inclusion of the word
granulomatosis means the new name recognises the history of
the disease name as well as a main feature of the pathology, and
the word polyangiitis both reects the frequent vasculitic involvement of multiple types of vessels and retains the nomenclature
used by the Chapel Hill Consensus Conference for vasculitic
involvement in a related condition called microscopic polyangiitis.7
The new term will not preclude its incorporation into a more

704

detailed revised nomenclature and classication scheme for the

vasculitides that may be developed in the future. Finally, we propose inclusion of the parenthetical term (Wegeners) for several
years to help smooth the adoption of the new name, avoid confusion in the medical literature and facilitate electronic searches.
Changing a disease name is never easy. We believe that the
wider medical and patient communities will accept and adopt
the name granulomatosis with polyangiitis (Wegeners) with the
same spirit of international and multispecialty cooperation that
led to our arriving at the new name.

Ronald J Falk,1 Wolfgang L Gross,2 Loc Guillevin,3

Gary Hoffman,4 David R W Jayne,5 J Charles Jennette,6
Cees G M Kallenberg,7 Raashid Luqmani,8 Alfred D Mahr,9
Eric L Matteson,10 Peter A Merkel,11 Ulrich Specks,12
Richard Watts13
1Department

of Internal Medicine, University of North Carolina, Chapel Hill, NC, USA

of Rheumatology, University at Lbeck, Lbeck, Germany
3Department of Internal Medicine, Universit Paris Descartes, and Hpital Cochin,
Assistance Publique-Hpitaux de Paris, Paris, France
4Department of Rheumatologic and Immunologic Diseases, Cleveland Clinic
Foundation, Cleveland, OH, USA
5Department of Nephrology, Addenbrookes Hospital, Cambridge, UK
6Department of Pathology and Laboratory Medicine, University of North Carolina,
Chapel Hill, NC, USA
7Department of Rheumatology & Clinical Immunology, University Medical Center
Groningen, University of Groningen, The Netherlands
8Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences,
University of Oxford, Oxford, UK
9Department of Internal Medicine Universit Paris Diderot - Paris 7, and Hpital
Saint-Louis, Assistance Publique-Hpitaux de Paris, Paris, France
10Departments of Medicine and Health Sciences Research, Mayo Clinic College of
Medicine, Rochester, MN, USA
11Department of Medicine, Boston University School of Medicine, Boston, MA, USA
12Department of Medicine, Mayo Clinic, Rochester, MN, USA
13Department of Rheumatology, Ipswich Hospital NHS Trust, Ipswich, UK; and
Norwich Medical School, Norwich, UK
2Department

Correspondence to Peter A Merkel, Boston University School of Medicine,

72 East Concord Street, Boston, MA 02118, USA; pmerkel@bu.edu
To be simultaneously published in Arthritis & Rheumatism, Journal of the American
Society of Nephrology and Annals of the Rheumatic Diseases
Provenance and peer review Not commissioned; not externally peer reviewed.
Accepted 31 January 2011
Ann Rheum Dis 2011;70:704. doi:10.1136/ard.2011.150714

REFERENCES
1. Woywodt A, Haubitz M, Haller H, et al. Wegeners granulomatosis. Lancet
2006;367:13626.
2. Klinger H. Grenzformen der Periarteriitis Nodosa. Frankf Z Pathol 1931;42:45580.
3. Wegener F. Ueber generalisierte septische Geferkrankungen. Verh Deut Pathol Ges
1936;29:20210.
4. Wegener F. Ueber eine eigenartige rhinogene Granulomatose mit besonderer
Beteiligung des Arteriensystems und der Nieren. Beitr Pathol Anat 1939;102:3068.
5. Godman GC, Churg J. Wegeners granulomatosis: pathology and review of the
literature. AMA Arch Pathol 1954;58:53353.
6. Falk RJ, Jennette JC. ANCA disease: where is this field heading? J Am Soc Nephrol
2010;21:74552.
7. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides.
Proposal of an international consensus conference. Arthritis Rheum 1994;37:18792.

Ann Rheum Dis April 2011 Vol 70 No 4

Downloaded from ard.bmj.com on May 17, 2012 - Published by group.bmj.com

Granulomatosis with polyangiitis

(Wegener's): An alternative name for
Wegener's granulomatosis
Ronald J Falk, Wolfgang L Gross, Loc Guillevin, et al.
Ann Rheum Dis 2011 70: 704

doi: 10.1136/ard.2011.150714

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References

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